Case report: Hemophagocytic Syndrome, clinical manifestations and diagnosis
DOI:
https://doi.org/10.61651/rped.2021v73n2p25-30Keywords:
Lymphohistiocytosis, Hemophagocytic, DiagnosisAbstract
Hemophagocytic syndrome is an inflammation with an inefficient, excessive and prolonged immune response. Characterized by fever, cytopenias, splenomegaly, bone marrow hemophagocytosis, hypertriglyceridemia and hypofibrinogenemia resulting from dysfunction of natural killer cells (NK) that leads to overstimulation, proliferation and ectopic migration of T cells.
The case of a 13-year-old male teenager from Huancavelica is described with clinical manifestations: weight loss of six in three months, jaundice, a history of fever, hepatosplenomegaly and pancytopenia. Large amounts of histiocytes with hemophagocytosis, positive viral load for EBV, relatives with family cultural contrast that delayed the etiological diagnosis and late treatment onset, unfavourable evolution, multi organic failure, enter to mechanical ventilation and death at 28 days of hospitalization.
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